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Biomedical subjects

A Bensman

Publications and source records attributed to A Bensman.

At least 37 records · Page 2Linked to original sources
PubMed1996

[Digestive manifestations in hemolytic uremic syndrome in children].

BACKGROUND: Gastro-intestinal manifestations are relatively frequent during the course of hemolytic uremic syndrome (HUS), some of them requiring special supportive care. This work was aimed at retrospectively studing gastrointestinal manifestations of HUS and determining their place in the prognosis. PATIENTS: Thirty-seven children aged 4 months to 11 years (22 girls and 15 boys) were included in the study. RESULTS: All children but one had gastrointestinal prodromes. During the course of HUS, various manifestations were seen: bloody diarrhea in 32% of patients, ileo-ileal intussusception in 3%, rectal prolapse in 8% and hepatic cytolysis in 38%. Seven patients with bloody diarrhea had a complicated course, lethal in one. Comparison between these seven children and the 30 others revealed some indicators of severe gut involvement: female sex, short duration of gastrointestinal prodromes, hemorrhagic colitis with rectal prolapse, high WBC count, high neutrophils count and less important degree of anemia at admission. Severity of the gastrointestinal lesions was correlated with that of the outcome of the renal disease. CONCLUSION: Gastrointestinal tract is frequently affected in HUS and severe complications can appear, potentially leading to death. Total parenteral nutrition could prevent occurrence of gastrointestinal complications. Severe gastrointestinal lesions are associated with a poor renal outcome.

Child↗
PubMed1996

[Prognosis of prenatal uropathies: should fetal urine tests be continued?].

Twenty percent of the children with end stage renal failure have severe obstructive uropathies. The assessment of fetal renal function and the degree of renal damage in utero is a critical factor in providing realistic counseling. The prediction of postnatal renal function is made by sonography, fetal urinary biochemistry and fetal serum for beta-2-microglobulin. Ultrasonography is a useful method including assessment of amniotic volume and ultrasonographic evaluation of the renal parenchyma. The analysis of fetal urine for the assessment of renal function leads to conflicting results. Some papers conclude that fetal urine electrolytes are not an accurate predictor of neonatal renal function, others conclude that they provide useful information. The number of studies dealing with the urinary beta-2-microglobulin levels in the assessment of prenatal renal function is too small for allowing definitive conclusions. The predictive value of fetal serum beta-2-microglobulin for neonatal renal function may be interesting but the results are yet preliminary. The studies of fetal urinary biochemistry should be continued within prospective research programs; the data are either too conflicting or too preliminary to use this methodology in commun practise.

Congenital Abnormalities↗
PubMed1995

Azathioprine-induced pulmonary haemorrhage in a child after renal transplantation.

We report a case of azathioprine-induced haemorrhagic alveolitis, in a 14-year-old boy, after renal transplantation. On day 25 the patient developed haemoptysis, fever and hypoxaemia. Chest X-ray showed diffuse reticulo-nodular shadows in both lung fields. Bronchoalveolar lavage samples were haemorrhagic and demonstrated a relative neutrophilia and a mild lymphocytosis, with a normal CD4/CD8 ratio. Azathioprine was discontinued on day 26. The patient required mechanical ventilation for 4 days. A positive leucocyte migration inhibition test and the recurrence of the symptoms after a second short course of azathioprine therapy suggested a cell-mediated mechanism. This patient is, to our knowledge, the first child to suffer from azathioprine-induced pulmonary haemorrhage.

Acute Disease↗
PubMed1995

[Transient correction of partial congenital factor V deficiency in nephrotic syndrome].

Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.

Child, Preschool↗
PubMed1995

Energy balance in children and young adults receiving haemodialysis for chronic renal failure.

This study was designed to determine the contribution of energy expenditure to the energy imbalance seen in uraemic children. Resting energy expenditure (REE) was measured using open-circuit indirect calorimetry in eight uraemic haemodialysed subjects aged 9.3-20.4 years and in 10 healthy children. Linear correlations between REE and both body weight and fat-free mass as measured by anthropometry were found in both controls and uraemic subjects (respectively: r = 0.76 and r = 0.88 for body weight and r = 0.73 and r = 0.90 for fat-free mass). Measured REE in uraemic patients was not different from the value predicted by using actual body weight and fat-free mass in the regression equation of REE on body weight and fat-free mass in controls (paired t test: p = 0.70 and p = 0.19 respectively). These data suggest that the energy imbalance seen in uraemic children is not due to increased energy expenditure and is therefore probably due to decreased food intake.

Journal Article↗
PubMed1995

Selection of transplantation procedures and perioperative management in primary hyperoxaluria type 1.

This paper outlines the different options of transplant procedures in patients with primary hyperoxaluria type 1. Isolated kidney, isolated liver and combined liver-kidney grafting are discussed. Combined liver-kidney grafting appears to be the preferred treatment for patients already in end-stage renal failure. The potential value of the two other procedures is outlined. Guidelines for perioperative care are given. These involve fluid regime, pyridoxine supplementation, immunosuppression and administration of crystallization inhibitors such as phosphate and citrate. Special emphasis is put on selection of appropriate dialysis procedures and reasons why haemodialysis and continuous haemodiafiltration are the methods of choice.

Fluid Therapy↗
PubMed1994

[Acute urine retention: a rare mode of revelation of cervico-dorsal syringomyelia caused by cyproheptadine].

BACKGROUND: Syringomyelia is rare in children aged less than 10 years, and bladder dysfunction is an unlikely first manifestation. This report describes a case of repeated episodes of acute urinary retention in a young girl revealing syringomyelia and Arnold-Chiari malformation. CASE REPORT: A 2.5 year-old girl was admitted because she was suffering from acute urinary retention. Her poor appetite had been treated with cyproheptadine, a histamine type I blocking drug. Clinical investigation revealed no local cause for this bladder dysfunction except moderate spasticity of the legs. Cystography showed no vesicoureteral reflux. Because the episodes of urinary retention recurred each day, magnetic resonance imaging (MRI) was performed; this showed the typical features of syringomyelia extending from C5 to T11 plus Arnold-Chiari malformation. The cyproheptadine was discontinued and the urinary retention disappeared. CONCLUSION: Cyproheptadine may have revealed latent neurogenic bladder in this case, although urodynamic studies, performed 3 months later, detected no bladder dysfunction.

Acute Disease↗
PubMed1993

[Treatment of urinary infection in children].

Urinary tract infection in children includes several and totally different diseases which must be managed according to circumstances. In all cases of acute pyelonephritis the patients must be investigated for abnormalities of urinary tract structures. A strong and prolonged treatment reduces the risk of scars. Lower urinary tract infection without potential deleterious effects on the renal parenchyma requires an oral antimicrobial therapy of not more than 7 days duration. Asymptomatic infection with normal urinary tract structures does not need an antimicrobial treatment. Long-term prophylactic antimicrobial treatment administered in full doses produces bacterial selection. Very low doses are sufficient to reduce the virulence of the pathogen and the risk of infection, without altering the gastrointestinal flora.

Acute Disease↗
PubMed1993

Collagen type III glomerulopathy: a new type of hereditary nephropathy.

A new type of hereditary glomerulopathy was observed in ten children presenting with early and progressive glomerular symptoms, often associated with hypertension. Light microscopy showed a diffuse increase in the mesangial matrix and generalized widening of the capillary walls. Electron-microscopic examination of renal tissue, after phosphotungstic acid treatment, revealed the presence of fibrillar collagen within the mesangial matrix and the subendothelial aspect of the glomerular basement membrane, adjacent to normal lamina densa. Immunohistochemical studies identified the fibrillar collagen not usually present within the glomerular extracellular matrix as type III collagen. Clinical and family studies ruled out the diagnosis of nail-patella syndrome, an autosomal dominant disorder with typical extrarenal symptoms, which is also characterized by the presence of fibrillar collagen within the glomerular basement membranes. The poor renal outcome, the possible extrarenal haematological and pulmonary involvement and the transmission as an autosomal recessive trait strongly suggest that collagen type III glomerulopathy is a new type of hereditary disease. From the high incidence of superimposed haemolytic uraemic syndrome in patients or their siblings, it may be hypothesized that collagen type III glomerulopathy is the underlying defect in some of the familial cases of haemolytic uraemic syndromes.

Adolescent↗
PubMed1993

[A rare cause of bone pain in children: primary hyperparathyroidism caused by adenoma].

BACKGROUND: Primary hyperparathyroidism is rare in children and adolescents. Bone changes may produce pain in the back or extremities, but this is rarely the first symptom of the disease. CASE REPORT: A 13 year-old girl suffered from pain in the back and lower extremities. Progressive bilateral genu valgum appeared. One year later, she became lame because of the deformity. ECG showed cardiac arrhythmia with atrial extra systoles. The serum calcium concentrations were 3.36 and 3.8 mmol/l, phosphate 0.76 mmol/l and alkaline phosphatases 6,612 U/I (N: 90-300). Urinary excretion of calcium was 17 and 26 mg/kg/day and the renal tubular reabsorption of phosphate was 77%. Radiological studies revealed resorption of subperiosteal bone, best seen along the margins of the phalanges, demineralization of the skull vault, bilateral coxa vara and zones of calcification on knee metaphyses. The serum concentration of parathyroid hormone (PTH) was 1,066 pg/ml (N: 10-55) and that of 1-25(OH)2D3 was 125 ng/ml (N: 20-80). Ultrasonography showed a heterogeneous mass, 23 x 15 mm, suggesting a parathyroid adenoma. This adenoma was independent of the left inferior parathyroid. It was removed and the biochemical findings gradually returned to normal. Bone demineralization also disappeared and the knee deformities were surgically corrected 9 months later. CONCLUSIONS: Bone changes may occasionally cause severe pain, indicating demineralization and hypercalcemia: hyperparathyroidism is one cause of such changes.

Adenoma↗
PubMed1992

[Neurectodermal malignant tumor of the soft tissues after treatment with immunosuppressive agents of lipoid nephrosis].

Immunosuppressive drugs are known to increase the risk of inducing neoplasia, especially acute leukaemia when high doses are used. A case of nephrosis in a 10 year-old boy treated with chlormethine (cumulative dose: 0.8 mg/kg) and chlorambucil (cumulative dose: 10 mg/kg) is reported. Four years after the beginning of the treatment an extraskeletal Ewing's sarcoma occurred. Since the review of literature failed to find any malignancy induced by such an immunosuppressive treatment for nephrosis, the question whether or not this extraskeletal Ewing's sarcoma was attributable to this treatment remains unanswered.

Adrenal Cortex Hormones↗